Biliary Pseudocyst: A Rare Complication of Antenatal Perforation of Forme Fruste Choledochal Cyst.

Spontaneous perforation of choledochal cyst (CDC) is rare with an incidence of 1.8%-7%. Perforation with pseudocyst formation is even rarer, there being only two case reports. We report the first case of antenatal perforation of CDC with biliary pseudocyst formation in a newborn and its management. Copyright:

INTRODUCTION

Spontaneous perforation of choledochal cyst (CDC) is rare with an incidence of 1.8%–7%.[1] Perforation with pseudocyst formation is even rarer, there being only two case reports. There is only one report of antenatal perforation of bile ducts, resulting in biliary ascites in the neonatal period. We hereby report the first case of antenatal perforation of CDC with pseudocyst formation in a neonate.

CASE REPORT

A male baby was born at term with gross abdominal distension at birth. Ultrasonography (USG) abdomen done on day 2 of life revealed two large cysts in the retroperitoneum, and a diagnosis of lymphangioma was made at a peripheral health-care facility based on USG findings. Right cyst was aspirated and sodium tetradecyl sulfate was injected into the cyst. In the next 2 weeks, there was re-accumulation of fluid in the cyst and a repeat tap with sclerotherapy was done again. He presented to us at 3 weeks of life with gross abdominal distension, poor feeding, fever, acholic stools, and failure to gain weight. On examination, the child was sick looking, was febrile, had icterus, and had signs of dehydration. Abdomen was grossly distended and tender and two large cystic mass were palpable, one in the left hypochondrium and left lumbar region (10 cm × 7 cm) while another mass in the right hypochondrium (5 cm × 5 cm). Rest of the systemic examination was normal. He was admitted in the pediatric intensive care unit and started with supportive treatment. Initial investigations revealed leukocytosis (40,000/mcl), hyponatremia (127 mEq/l), conjugated hyperbilirubinemia (TSB/DSB: 5/3.4 mg/dl), transaminitis (SGOT/SGPT: 441/292 IU/l), raised GGT (129), and hypoalbuminemia (1.18 g/gl). USG abdomen showed two large cysts, one on the left and other on the right in the upper abdomen. A diagnostic tap was done which revealed bilious fluid. Computed tomography abdomen confirmed the cystic collections with a possibility of lymphangioma [Figure 1]. The child was explored after a course of intravenous antibiotics. Intraoperatively, two large pseudocysts were seen, one in the lesser sac (approximately 600 cc) and the other (approximately 400 cc) on the right communicating with the common hepatic duct (CHD) [Figure 2]. Both the pseudocysts were inter-communicating through the foramen of Winslow. A perforation was identified in the CHD and terminal CHD was found to be dilated. Common bile duct (CBD) was fibrotic. Both the pseudocysts were excised and the hepatic duct was drained out through a tube drain. Six weeks later, re-exploration and hepaticojejunostomy were performed with uneventful recovery. The child is well on 3 months of follow-up; is accepting feeds well, gaining weight, and passing pigmented stools; and has no jaundice.

Figure 1

Computed tomographic scan of the abdomen showing intra-abdominal collection

Figure 2

Intraoperative picture showing both the pseudocysts

DISCUSSION

CDCs are congenital cystic dilatation of the intrahepatic or extrahepatic biliary tree. The incidence of biliary cysts in the Western population is 1:100,000–1:150,000.[2] The incidence is higher in some Asian countries (1:1000).[3] They are more common in females with a female-to-male ratio of 3:1–4:1.

CDC presents differently in each age group. Majority of them presents before the age of 10 years. The classic presentation of abdominal pain, jaundice, and palpable mass is more common in children than in adults. In the neonatal period, it typically presents with obstructive jaundice and abdominal masses. In our case, the baby had an antenatal perforation as was evident from the baby being born with massive abdominal distension and confirmation of two large cysts on USG on day 2 of life. On exploration, the left pseudocyst was easily dissected, but on the right, there were adhesions because of previous sclerotherapy. The distal duct was completely replaced by a fibrotic cord, and there were some remnants hanging on the CHD at the site of perforation. Had it been a spontaneous biliary perforation, then the distal CBD should have been normal and not fibrotic. CDCs are diagnosed both antenatally and in the neonatal period by ultrasound screening. They can be diagnosed as early as 15 weeks of gestation even in the absence of fetal enzyme production. CDCs in the neonatal period and early infancy tend to present with distal obstruction of the bile duct as a result of which there is obstructive jaundice, cyst enlargement, or rupture. The complications of CDC are cyst enlargement, accumulation of biliary sludge, pancreatitis, spontaneous perforation, and pseudocyst formation. Perforation of CDC reported is 1%–12%. The incidence of pseudocyst formation is even rarer, with only two cases reported.[45] The bile duct wall lacks elastic fibers in neonates and in early infancy, and hence, CDCs are more susceptible to perforation. The site of perforation is most commonly at the junction of cystic duct and CBD. The most common site for biliary pseudocyst formation is adjacent to the site of perforation. Primary cyst excision with biliary reconstruction is the surgical procedure of choice. This can be done in a single stage or in two stages. This case has been described as a forme fruste CDC because there was minimal dilatation of the extrahepatic ducts.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parent has given his consent for his images and other clinical information to be reported in the journal. The patient's parent understands that his names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Consent

Written informed consent was obtained from the patient for publication of this case and any accompanying images.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.